Disease course patterns in systemic lupus erythematosus

Abstract

Background Disease activity in systemic lupus erythematosus follows three different courses: long quiescent, relapsing remitting and persistently active. However, the patterns of disease course since diagnosis are not known. This study aimed to assess the prevalence and characteristics of such patterns over 10 years. Patients and methods The inception cohort of the Toronto Lupus Clinic (≥10 year follow up, between visit interval ≤18 months) was investigated. Prolonged remission was defined as a clinical Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 achieved within 5 years of enrolment and maintained for ≥10 years. The relapsing-remitting pattern was defined based on ≥2 remission periods (clinical Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 for two consecutive visits). Patients with no remission were categorized as persistently active. Groups were compared for baseline characteristics, cumulative damage, flare rate, mortality and certain co-morbidities. Results Of 267 patients, 27 (10.1%) achieved prolonged remission, 180 (67.4%) relapsing-remitting and 25 (9.4%) persistently active. In total, 35 (13.1%) had only one remission period (hybrid). At enrollment, there were no differences regarding clinical and immunological variables. At 10 years, persistently active patients had accumulated significantly more damage than the prolonged remission and relapsing-remitting patients. Being of Black race and higher adjusted mean Systemic Lupus Erythematosus Disease Activity Index 2000 over the first 2 years were associated with a more severe disease course. Relapsing-remitting and persistently active patients had an increased flare rate and accrued more osteoporosis, osteonecrosis and cardiovascular events. Conclusions Approximately 70% of systemic lupus erythematosus patients followed a relapsing-remitting course, whereas 10% displayed prolonged remission and another 10% a persistently active course. Early response to treatment was associated with a less severe course and better prognosis.

The baseline interferon signature predicts disease severity over the subsequent 5 years in systemic lupus erythematosus

“…It is possible that at disease inception the levels of IFNinduced gene expression may not be as informative. Studies show that following disease onset patients can follow one of three disease courses, persistent disease activity, recurrent remission and exacerbation, and initial disease activity followed by prolonged disease quiescence [33]. Whether these courses can be predicted by the levels of IFN-induced gene expression at disease inception remains to be seen.…”

Epstein-Barr Virus and Systemic Autoimmune Diseases

“…SLE is a disease, which clinically presents with a heterogenous array of symptoms, often evaluated by the SLE disease activity index (SLEDAI) or similar indexes, including complementemia, DNA Abs, leukopenia, thrombocytemia, fever, fatigue, skin rash, UV sensitivity, mucosal ulcers, alopecia, pleuriris or pericarditis, proteinuria, hematuria, nephritis, myositis, arthritis, vasculitis, headache, stroke, and more rarely, neuropsychiatric symptoms (134)(135)(136)(137). The disease may show a relapsing/remitting course, depending on the efficacy of treatments (138,139).…”

Remission in systemic lupus erythematosus: testing different definitions in a large multicentre cohort

“…(HRQoL)) [1][2][3][4][5][6][7][8][9] ; however, a universally accepted definition of remission combining evidence-based medicine and expert opinion is still missing. 3 10 11 The Definitions Of Remission In SLE (DORIS) international task force defined remission as a sustained state without any symptoms and signs of SLE, assessing disease activity according to the clinical SLE Disease Activity Index 2000 (cSLEDAI), 10 which does not take into account serology, 12 and evaluating the global patient status by Physician Global Assessment (PGA).…”